Proteomic analysis of spinal cord of presymptomatic amyotrophic lateral sclerosis G93A SOD1 mouse.

Abstract:

:Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease, whose primary mechanisms or causes are still not defined and for which no effective treatment is available. We have recently reported that before disease onset the level of tyrosine nitrated proteins is increased in the G93A SOD1 transgenic mouse model of ALS. In the present investigation, we carried out a proteomic analysis of spinal cord extracts from G93A SOD1 mice at the presymptomatic stage of the disease to further unravel primary events in the pathogenesis and tentatively screen for potential pharmacological targets. Using a robust two-dimensional gel electrophoresis-based proteomic approach, we detected a number of proteins differentially represented in presymptomatic mice in comparison with controls. Alterations of these proteins correlate with mitochondrial dysfunction, aggregation, and stress response. Moreover, we found a variation in the isoform pattern of cyclophilin A, a molecular chaperone that protects cells from the oxidative stress.

authors

Massignan T,Casoni F,Basso M,Stefanazzi P,Biasini E,Tortarolo M,Salmona M,Gianazza E,Bendotti C,Bonetto V

doi

10.1016/j.bbrc.2006.12.075

subject

Has Abstract

pub_date

2007-02-16 00:00:00

pages

719-25

issue

3

eissn

0006-291X

issn

1090-2104

pii

S0006-291X(06)02752-5

journal_volume

353

pub_type

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